Go girls...

Elsa is doing incredible. She is now pulling herself to a stand and her crawling has become much better. I found her standing in her crib when I went to get her from her nap yesterday. Today, she repeatedly pulled herself to a stand using the ottoman to try to get Sophie's sippy cup that was sitting on top of it. It was so adorable. As soon as she would stand up, she would fall over. But she kept trying and trying until she fell pretty hard and started crying. All that practice paid off because by the end of the night, she was standing pretty steadily.

Sophie is talking more and more each day. She is using sign language all the time now without being prompted and her word approximations are becoming more clear. Her newest words are dog, star and open (all are approximations but are close). She is also much more stable and climbing up anything and everything. Her mood is so much better and she has lost a couple of the pounds she gained during ACTH.

Haley tried out for dance competition and made it onto 5 different competition lines (though she only accepted 4 so she could have an extra night off during the school week). She did so well in her competition lines last year with winning a double platinum in one of them. She also started school last week and will again be in the Quest program (a gifted program in which she was accepted into for scoring so high on a special test she took). It amazes me how incredibly smart she is.

I am so proud of all my girls!!!

When you least except it...

I had a blast in Gatlinburg, Tennessee. I have to admit...I was not looking forward to this trip. I had knots in my stomach, not able to sleep, small panic attack kind of feelings towards this trip. Sophie was just coming off ACTH (her last shot was the night before we left) so my emotions were already running high from the whole drama of what we were going through at the moment. I was worried about her weakened immune system. There was no being able to stop off at some restaurant along the way and stretch our legs or burn some energy. Sophie couldn't be in public places. I knew Sophie would be fine in the car but Elsa was a whole different story. Elsa is on the go and does not like to be strapped down. I can barely even hold her at times without her wiggling around like crazy. Then once we got there, what was I suppose to do with an immune suppressed 2 year old and a 6 month old. Little did I know...

Not all of us did all of these since we took turns staying back with Sophie and Elsa at times but if we could take them we did...white water rafting, hiking to Laurel Falls, swimming (lots and lots of swimming), Ripley's Aquarium, Ripley's Believe It or Not! Museum, Ripley's Mirror Maze, go-karts, putt-putt golf, regular golf, Cirque de Chine (simply amazing), Pancake Pantry* (best pancakes ever), Ober-Gatlinburg (mini outdoor amusement park), and that is all my mind can remember at the moment.

Ober-Gatlinburg
The faster the ride, the steeper the slide, the more fun Sophie had...she has no fear!

After the ride was over, Sophie would point to the slide and say "moh". She had so much fun.


Those gorgeous eyes...yet I can't help but notice the 2 scars...one above each eye.

Notice the mountains in the background...our view was absolutely gorgeous.

We saw several bears. This one came right through our backyard.

Laurel Falls
Sophie fell asleep on the walk up.

The water was really cold but Elsa loved putting her feet in it.

Haley and Brandon climb a little higher which made me nervous...that's why there are dads in the world.

*If you ever been to Gatlinburg, you would know why I mentioned which pancake house we ate at...I have never been to a place that had a pancake house on every corner...literally.

Go Elsa!!!

Elsa is just amazing. Though I don't know if I would truly appreciate all she is doing if it wasn't for Sophie. So I feel I have the right to brag. That I get to be exempt from being classified as one of those moms that constantly brags about their exceptional child that can be so annoying especially if you never had kids. So here goes... Elsa is now 6 months and 3 weeks old...exact age is very important ;) In the past week, Elsa has taught herself to transition from laying to sitting, crawl*, blow raspberries and pull herself up to a high kneel. She is so alert and aware of her surroundings. She is very determined. If you move something out of the way that she shouldn't be playing with, she will follow it with her eyes and watch where you put it and then try to get it. If she drops something while sitting in her highchair, she will search for it. No "out of sight...out of mind" for her. It is so much fun watching her grow.

July 4, 2008 - 5.5 months old

*okay she is barely crawling...just a few forward movements but she can definitely go backwards

We are done!!!

No more ACTH. Actually, Sophie's last shot was last Friday. It has been crazy busy around here between figuring out what to do next with Sophie's treatment options and getting ready for vacation. We are currently in Gatlinburg, Tennessee. We left last Saturday and we are all having a blast. The ride down went rather smooth. We made our first stop about 3 hours outside of St. Louis at Paducah, Kentucky to visit some friends (Dana, Scott and Brock). Since Sophie's immune system is currently almost nonexistent...going into public places is a gamble. So we relied on friends and family on our trip down with a overnight stop in Nashville, Tennessee at Brandon's cousin Mike's house. So about 34 hours later (only about 9 or 10 hours in the car), we arrived at our rental house in the Smoky Mountains. More to come...

Ughhh...

What sucks more than giving your daughter daily injections of ACTH? Having to still give her daily injections when it did NOT work. Ughhhhhhhhhhh!!!!!!!!!!!!!! Her seizures are slowly creeping back up in numbers. Ughhhhhhhhh!!!!!!!!!!!!! What's next? Who knows. After failing 12 treatments (8 different drugs with trying 2 twice, ketogenic diet, IVIG), it is hard to think up another option. When do you say enough is enough.

I talked with her neuro on the phone the other night. He said he doesn't think anything is going to stop her seizures. He said it is just trying to find a balance of keeping them somewhat under control while minimizing the effects to her development. When I asked if they could possibly morph into a different type of seizure that would be easier to control (I am trying to hold on to a little bit of hope here not really trying to have him predict the future but knowing the personality of my neuro I do not know why I ask him these types of questions)...he says...hopefully but they might not be able to be controlled either. Why...why do I ask him these things. I really need to find a new neuro. I need a neuro that says maybe we will find something...maybe she will outgrown them...just something to keep me hoping. But maybe I should let go of that hope. Just enjoy Sophie day to day. Stop thinking about her future. But what parent doesn't think of their child's future...special needs or typical developing. As a parent, that is hard to do. I never thought about her having uncontrollable grand mal seizures...until now. I should be happy that she is only having uncontrollable tonic seizures and infantile spasms. It could be worse. I should really stop posting so late at night. I am really depressing at this hour. Sorry. So much for my new philosophy of thinking positive. Tomorrow is a new day. I will try again tomorrow. Think positive. I will keep telling myself that.

She's back...

Our sweet Sophie is back. She is back to her old self. Well almost. She is still eating a lot and she is still fairly chubby but she has actually lost 2 pounds and her face is not as round. But she is happy!!! She is playing independently, smiling, laughing, just enjoying herself.

She is talking up a storm. She is constantly making some kind of sound or another. Sometimes words...sometimes grunts. She is so big now that she grunts when she is trying to climb up on something. Her new words...

Door (she said door when I asked her to walk the nurse to the door)

Pop (Grandma Debbie taught her pop while she was snacking on popcorn)

Night-night (she is signing night-night a lot so whenever I see her sign night-night, I say night-night and she just said it one night...out of the blue just so clearly that I had to think for a minute to remember if she had ever said it before)

Pe-Bah (short for peanut butter...she said it while standing in front of the pantry but I haven't heard her say it again)

Ever since we started weaning ACTH (we are at .5ml once a day), her development started taking off again. It is so wonderful to see her so happy and joyful.

Label Cloud...

I'm playing around with Label Cloud so if you subscribe to my blog...sorry if it is sending you lots of e-mails. I'm not quite sure how that works if you republish old posts.

More on Elsa...

On the other hand, Elsa is doing absolutely wonderful. She is now 5 months old. She is sitting up independently. She is so active and determined. She can twist and turn her body in crazy ways in order to reach something she has her eye on. And she won't give up until she gets it. Brandon said the other day "should we be teaching Elsa on how to transition from laying to sitting". Sometimes she falls over and she gets so mad when she can't sit back up. But I said "not Elsa...Elsa gets to be just Elsa...she will learn on her own...she is already trying to figure out how all by herself". It is hard not to be in therapy mode sometimes. But I am enjoying taking a step back with Elsa and just watching. Watching her do so many things easily that Sophie has struggled with. It fills my heart with joy to just sit back and watch Elsa. I have learned from Sophie not to take these things for granted but to soak in every moment. The way Elsa lights up every time I pass in front of her view. The expression is priceless. The moment will always be cherished in my heart. The way Elsa is just so alert and explorative. Every sound needs to be checked out, every object needs to be looked over. She is just amazing.

Weaning...

Both neuros are in agreement...it's time to wean. If it hasn't worked now, it's most likely not going to. UGGGGHHHH!!! We had to try it. So we did. I can't look back now but I so hate what it did and still doing to her body and spirit. She just so miserable. VERY MISERABLE. Poor thing. I drove around in the car with her for at least an hour today. I didn't know what to do. All she wants to do is eat but she is tired of eating so she didn't want to eat but her body kept telling her she was hungry. I couldn't do anything to settle her down so I loaded the kids in the car and we went for a ride. Luckily, it was about Elsa's nap time so she slept the whole time. Sophie just watched her video. Soon this will all be over. The wean will take 4 weeks. I think we might try to shorten it to 3 weeks. Why not? It's not like it stopped her seizures. She is suppose to be on .5ml/day for 7 days, then .4ml/day for 7 days, then .25ml/day for 7 days, then .1ml/day for 7 days. I think we might do each dose for 5 days instead. I just so want to get this over with.

What to do???

Sophie is on Day 26 of ACTH...only 37 more days to go. ONLY 37 more days!!! Seems like a long time. Brandon and I have a pretty good system down on giving her the shot but it stills isn't any easier emotionally. Especially since we are still on twice a day (for another 9 days).

Her seizures haven't gone away. They are better but they are still there. She is not having as many and they are much more milder. She hasn't had one of the "ones that makes her cry" seizure for quite some time now. It is encouraging that they are better but what does this mean for her. She is absolutely miserable. Always hungry. Always tired. Huge. Uncomfortable. So even though her seizures are better, she is too miserable to try to learn anything new. It is such a struggle to get her to even smile. Her two favorite signs at the moment..."eat" and "night-night". Every time I lay her down to change her diaper, she signs "night-night".

Flashback 2 years and 2 months ago...Sophie started her first round of ACTH (May 11, 2006). Her seizures got better. They were much more mild. But they never completely went away. Once we weaned the ACTH, they came back with a vengeance. They slowly crept up to over a 100 a day.

If the ACTH was going to completely stop her seizures, they most likely would have stopped by now. So now Brandon and I are debating on whether to continue the high dose for another 9 days or start weaning earlier. 9 days may not seem like a long time but when it relates to ACTH...it is a very long time. Especially with a miserable, very hungry, moody, tired, bloated 2 year old. I will be talking with both of Sophie's neuros tomorrow to see what their recommendations are.

There's more...

When I post, it is usually late at night. I am tired and my brain isn't functioning all that well. I try to get out the essentials and always tell myself that I will share all the good news in my next post but...

So here goes...

Sophie's expressive language continues to grow. She is becoming more and more spontaneous with her words and signs. She says things now that I completely have no idea what she is talking about but she is trying to find the words to tell me. It's different syllables put together that is suppose to be a word but I just haven't figured it out yet. But she is trying. She is trying to say more than just the words we ask her to repeat. That is such incredible progress. Her list of words continues to grow. She says "HeyHey" for Haley and a weird sounding "el" for Elsa. Oh, I just remembered that I left off "up" from the last list. She is also attempting to say "open".

Her walking continues to become more stable. She is going up and down the steps with more independence. Actually, she can walk up the steps all by herself now. Walking...no crawling. Sometimes it is even step over step (meaning only one foot on each step instead of putting one foot on the step and the other foot on the same step).

There's more but it is really late now and I really cannot think at the moment. I promise to share pictures and some sweet stories really soon.

Good day...

Sophie had a good day...as far as seizures go. Hip Hip Hooray!!! We are praying that this is it. That today was the turning point. That it just wasn't a fluke.

Sophie weighed in at 36 pounds tonight. Just two weeks ago she was 31.5 pounds. You can see it...in her face and in her belly. She is round. She REALLY doesn't look like my baby girl anymore. After Elsa was born, Sophie looked completely different to me. Laying next to Elsa, I could no longer look at her as my baby girl but my toddler girl. Her legs looked longer, her feet looked bigger. Now she has lost all the babiness she had left. She is just so big. So heavy. So incredibly heavy. And tired. It is so hard to keep her entertained during the day. It is just so hot outside. And if we are inside...she just wants to cuddle up next to me and suck her thumb and hold my hair. So we try to keep her busy. Keep her motivated to do something. We went swimming twice this past weekend. And Uncle Jason took her swimming yesterday. She LOVES swimming. She loves the water. She loves her bath.

Just when we were ready to give up on this drug. Ready to start weaning early. Things changed. It will be easier emotionally to give her the injections if we keep seeing such a positive change in her seizures. I have to believe this. I have to stay positive. Please keep praying for her.

Quick update...

Tomorrow we will increase the dose to .5mL (40 units) twice a day. No significant changes in her seizures. Some days are better than others. Some days I think it is working then the next day I question what did I get myself into. Even though it has been easier on Sophie to get the shots, it is still just as heartbreaking but we are all recovering much quicker. Sophie sees "the blanket", starts crying, I hold her down, Brandon gives the shot, one of us takes her outside to play on the swingset, everyone feels better all in the matter of minutes. Oh, and you better make sure you take her directly to the swingset. No trying to distract her with anything else. If you don't take the quickest path to the back door, she will redirect you quickly :)

By the way, we no longer have to swaddle her. She just lays there. We still have her lay on the blanket so she knows what is about to happen. I don't want her to look over her shoulder all day wondering if we are going to sneak up on her and stick her with a needle. Needless to say, we will be having a blanket burning party after this is all done. Hopefully, we can combine that with a seizure-free party.

One last thing...her neuro called today to check up on her. I gave him the update and he also had her lab results. Her blood sugar level was low. This could be for a number of reasons. Since it was taken last Wednesday, I can't remember what she ate for dinner the night before or if she had a bedtime snack. She is having her blood drawn tomorrow morning and we should have the results by tomorrow afternoon. We gave her a bedtime snack tonight so hopefully her labs results will be just fine this time around.

It's late...

It's late and I really shouldn't be posting on my blog at this hour. I am too emotional. Emotions and tiredness do not mix well. I am depressed at the moment. I try to be strong but at this moment I am not. I can't sleep. It's 12:30am and a nurse will be at my house in about 7 1/2 hours to administer Sophie another injection. These shots are wearing on me and even more so on Sophie. The thought of her not being able to go to preschool makes me so sad. She has made so much progress there. But she has much more progress to be made. But she is making progress so I should be happy about that, right? I am happy. I am ecstatic about it. I am just tired of being happy about it...if that makes sense??? It is just that I wish it would all come naturally. That it is not so much work. Everything Sophie does is analyzed 100 times over. Sophie's current therapist count...15 (currently she is only working with 9 different therapist). Sophie's current doctor count...4 pediatric neurologist in 3 different states, 3 orthotist in 2 different states, 2 pediatricians, 1 developmental pediatrician, 1 ENT, 1 ophthalmologist and 1 hematologist.

Then there is the guilt factor. Guilt that I am not working hard enough. Guilt that I am not grateful enough. Guilt that I am sad. How can I be sad when Sophie is considered one of the "lucky" ones?!?! How can I be sad when I am fortunate to get such incredible care for Sophie?!?! Guilt that I am even talking about myself. Guilt that I am even writing this post.

I am grateful. I do realize the "what ifs". That is why I feel guilty for feeling the way I feel.

The only good thing about this post is that Sophie is still sleeping. She made it past the midnight hour. That is when she usually wakes up (somewhere between 12am and 1am). So maybe I can sleep with some peace now...maybe I can stop holding my breath.

ACTH - Day 8

We increased the dosage today to .4mL twice a day. The nurse came this morning to draw blood* and give the shot and Jake came this evening to give the shot (thank you...I know how emotionally hard that must have been on you...you did a great job...she didn't point to the boo-boo on her leg afterwards like she sometimes does). Needless to say, Sophie was a little wiped out for school today. I wish I would have planned it better to where her increase in dosage was not on the morning before she went to school.

I want to say thank you to everyone for being so supportive through this process. Jason...thank you for coming over the first few days and assisting Brandon with the shots. My IS friends...thank you for all your support and advice...what would we do without the internet?!?! My mom...thank you for coming over during the day and helping out with the girls especially at a moments notice. My mother-in-law...thank you for always being there especially these last few days while Brandon has been out of town...I can't imagine what this morning would have been like if you were not here.

Seizure update... A bit better. No drastic changes but sometimes the seizures are a bit milder than usual. On Monday night, she slept through the entire night without waking up!!! It has been awhile since she has done that. It was absolutely wonderful. Elsa didn't wake up until 5am and then went right back to sleep. They both woke up around 8am. Thank goodness because this was the first time I have ever spent the night alone with both kids. Last night, she woke up at about midnight and had about 5 mild seizures. So her nights have been getting a lot better.


* The nurse was able to draw blood for lab work via a finger poke. Sophie had to hold this warm pack in her hands for a few minutes to increase blood flow and boy did it work. Her finger wouldn't stop bleeding for a bit. It bleed through the gauze pad and we had to put a second band-aid on her. I'll take this any day over trying to fish for a vein in the arm. There is only one nurse at the lab at the hospital where we usually go that can get it on the first try. So YEAH for the finger poke! And BOO for the fact we recognize the nurses who draw blood.

More words...

I left "more" and "wall" off the list. She also said "go" in therapy today. Oh, and she also says "ball" spontaneously. I knew I was forgetting something.

Talking...

Sophie is talking!!! In the mist of everything going on, I haven't had time to post about all the great things that have been happening around here lately. The words aren't that clear and there are probably only a handful of us that would understand her BUT she is trying. Trying very hard. Here's a list (somewhat in order of when she started saying them) and I am sure I am probably missing a few...

mama
ball
rock
moon
tree
dada
wawa (water)
yes
no
this
bird

I can't even explain how excited I am that she is talking. I use to wonder if she would ever talk. Then the sign language started and then I knew she would talk...it was just a matter of when. Now she is talking. Mostly the words are prompted (meaning "Sophie can you say...") but she has said two words spontaneously...mama and rock. Mama she said spontaneously when I picked her up from preschool a few weeks ago and now she says it spontaneously quite often. Rock she has said spontaneously only one time and that was when she walked by and saw one laying in her basket. It is a start. A great start!!!

ACTH - Day 3

The nurse gave Sophie her first shot which went okay...just a little blood and a lot of tears but she calmed down within a few minutes. Brandon gave Sophie her second shot which didn't go so well...a lot of blood and a lot of tears and she kept pointing to that spot on her leg all night long. Then Brandon gave Sophie her third shot last night...the best yet with no blood and a few tears and she calmed down quickly. So hopefully this will continue to get easier with time. Last night, after much advice from many different people (thank you all by the way), we decided to swaddle her and lay her down (the first 2 times I tried to hold her in my lap and wrap my arms and legs around her) and it was much easier to keep her still.

Thursday wasn't a very good day. She was very moody and had many seizures.

Friday was a better day. She was in a great mood and had her usual amount of seizures during the day but had a great evening until about 1am when she had a fairly large cluster of seizures that made her scream and cry.

This morning has been much better though and the total count so far is 7 (not including 3 movements that were questionable).

Currently, Sophie is on .5mL (40 units) given at night. On Wednesday, we increase to .4mL twice a day. The nurse will be coming on Monday, Wednesday and Friday mornings to give Sophie the shot so that will be a big help with Brandon's work schedule.

Pure torture...

Please share if you have any advice on how to hold down a 2 year old while she is screaming, crying and trying to wiggle her way out of getting an intramuscular shot. Oh, and any advice on how to stay emotionally detached since she is your own daughter? Is this going to get any easier? It is breaking my heart. I am tired of holding her down while she gets leads glued to her head for an EEG. I am tired of holding her down to get blood drawn or an IV started. I am tired of holding her still to get examined by doctors. All the while she screams and cries. I am suppose to protect her not hold her down while others do painful procedures.

So far, so good...

Two nights ago, Sophie woke up at midnight and had a bad cluster of seizures. They were strong and she had about 30. She was making strange arm movements and crying. She has actually been waking up in the middle of the night lately and having seizures. Well, last night, she woke up at 12:50am. She sat straight up in bed and started crying. It is hard to get her to lay back down. For some reason, laying back down upsets her. So I got her to cuddle up in my arms and she fell right back to sleep. It felt like she had about 6 myoclonic jerks which are different than her infantile spasms and tonic seizures. Myoclonic jerks are where your body just kind of shutters when you are falling asleep...can be perfectly normal. Her arms didn't thrust out. This is a great sign. Then she woke up at 6:30am this morning and Brandon lay down with her and she fell back to sleep without having any seizures. She is still sleeping. Her seizures are the worst in the middle of the night and first thing in the morning. Could the ACTH work this quickly?!?!

ACTH - Day 1

What a day!!! I am so glad it is over. I woke up this morning with big knots in my stomach. I was so dreading this day. The vials were in the fridge...everything was set up to start the treatment but I just wasn't ready. But would I ever be??? I just felt so overwhelmed, exhausted, stressed, sad... There is a list of things I want to do with Sophie this summer. So many things that I know she would enjoy. So many things I am now going to have to postpone. The thought of what this drug does to her just makes me feel so sick. I don't know why this drug over others. All the drugs have their side effects. I guess this one is just so visible. I see on the outside what it does to her body. The other drugs are just these little pills that she takes that hopefully will stop her seizures. I feel so bad for Brandon to have to give Sophie the injection and I feel so bad for Sophie to have to receive them. The other drugs are painless. Sophie loves taking her medication. I put all the pills on a plate and she just picks them up and pops them into her mouth like candy. I am so lucky that she is such a great pill taker.

Back to ACTH... the nurse arrived a little after 5pm. She went over all the paperwork and then it was onto the injection. I drew up the injection just like old times and handed it over to the nurse. I held Sophie down while the nurse gave the injection and Brandon watched. Sophie screamed and cried and tried to wiggle her way out of it. ACTH is a gel and it takes awhile to push all of it in. Then I immediately picked her up and we went outside to play on the swingset. I sat on a swing and she sat on my lap while I sang a song to her. She felt better in minutes. On the other hand, I just wanted to crawl up in a ball and cry. I just feel horrible. I just can't shake this awful feeling I have inside me. I have to take her out of preschool. She LOVES preschool. She gets so excited when I tell her in the morning that we are going. I hate that I have to take that away from her. But potentially I could be giving her so much more. Seizure freedom. Please pray that this will work.

ACTH update...

I miscalculated how many vials of ACTH Sophie is scheduled to get. It is actually 8 vials. I spoke with the pharmacist that will be shipping the ACTH to us since local pharmacies no longer deal with it. I asked her how much the insurance is paying for each vial of ACTH and she said $25,000. Then I asked how many vials are being shipped. She said 5 vials (the other 3 vials will be shipped later). That is $125,000 worth of drugs in one small package. Each vial is 5ml. Yes...only 5ml. 5ml is equivalent to 1 teaspoon. So 5 teaspoons of ACTH is equal to $125,000!!! This will be the most expensive package that I will every receive.

So I spent about 2 hours on the phone today going back and forth with the pharmacist, the nurse from the local children's hospital who will be coming out 3 times a week, and the nurse from our neurologist's office to get everything squared away. We are set to start ACTH next week. Actually as early as Monday but I think we will start Wednesday night. I feel for Brandon who will have to give Sophie the majority of the shots (the visiting nurse will give it when she is here). I am just not strong enough to do it. There will actually be a 4 week period when Sophie will have to get 2 injections a day. She will be on ACTH for a total of 9 weeks.

I pray that ACTH works quickly and that we never have to go on the highest dose.

Neuro appointment...

So we went in for our forever dreaded routine neuro appointment. They are so routine that Brandon doesn't even go with us anymore. It started out with Brandon going to all the appointments with us...to just getting conference in via speakerphone...to just getting a recap when he gets home from work. They all go something like this...

Neuro: What medications is she still on and what is the dosage?

Me: blah, blah, blah (update with her ever-changing medication schedule)

Neuro: How are her seizures?

Me: She is STILL having daily seizures. They are the worst when she wakes up in the morning.

Me: What do we try next?

Neuro: In so many words...It really doesn't matter what we try next because since she has failed so many medications that there is a very slim chance anything will work and if anything does work then it probably is only a matter of time before they come back.

Me: Well, then what has the least effect on her cognition because her development is INCREDIBLE right now. Then I go down the whole list of all the new things she is doing.

Neuro: No reaction. NOTHING, NADA, ZILCH.

Neuro: We can try... (fill in with yet another medication to try).

Me: Okay. Thanks for your time.

Some might wonder why we stick with this neuro but he has been with us since day 1. Even though he is not Mr. Personality, I still like him. He has always been open to any suggestions I might have and he is always willing to consult with other neuros. Not to mention that he is a pediatric neurologist...not an easy title to earn.

So what makes this appointment different than the rest is as follows...

I occasionally keep in contact with the neuro in Detroit that specializes in Infantile Spasms via e-mail. Well, this time he responded to my e-mail with a phone call. So I went through Sophie's history of medications and treatments we have tried since we last saw him and he suggested a second round of ACTH. Yes, that is right...a second round of ACTH!!! Ugh!!! The side effects to her body are hard to watch but this was the most successful drug she has been on. This was the closest to her becoming seizure-free from any drug (we still do not know why she was seizure-free for 33 days back in December 2007/January 2008).

So I mentioned this to her neuro during her last appointment. He called the neuro in Detroit. All is in agreement that Sophie should do a second round of ACTH. So that is what we are going to do. We will be starting the treatment in the next couple of weeks. Everything has been approved by insurance which is huge considering each vial is about $23,000 and she will probably go through 5 vials while on this treatment. The cost is insane - right?!?! The cost of ACTH during Sophie's first round of treatment was about $2,000 a vial.

So, that about sums up our last appointment. The appointments are a necessary evil...always leaving with an overwhelming feeling of something that I can never quite pinpoint but it is never good. How can it be good??? Sophie is still having seizures :(

Balls...

Sophie loves balls. She loves saying the word ball, she loves kicking the ball, she loves throwing the ball...anything to do with a ball. I don't know if it is because she can say the word or if she said the word because she loves the ball.

Depakote and seizures...

We are now down to one Depakote capsule a day (125mg). Her seizures seemed to have stabilized. She probably has anywhere from 30 to 50 seizures a day. It's hard to keep track. Or really, I just hate counting them. I use to keep a log of every seizure she had. I would write down when, how many, what type. I just can't keep up with that anymore. Now, I pick a day and I'll record them and then forget about it for awhile. I tried to track them today since we have her neuro appointment tomorrow, but I didn't. Let me try to recall...she had about 8 when she woke up this morning...then a half hour went by and she had a few more...then she had a few periodically throughout the morning...then she had about 10 when she woke up from her nap...then she had a few periodically throughout the evening...probably around 30 in total...and those are only the ones I saw. After 2 years of having seizures, this is what it has come down to. It is just part of our daily lives. So I will try to post tomorrow and let you all know how her neuro appointment goes.

Elsa, Elsa, Elsa...

Yesterday, Elsa rolled over from her back to her tummy for the first time (in the past it was always from her tummy to her back).

She loves chewing on her toes. Actually, she loves chewing on anything she can get her hands on. I can't even take a drink of water while holding her without her trying to grab the cup.

I just can't get over how alert she is. She is constantly following Sophie with her eyes. They are just so adorable together.

On Wednesday, she has her 4 month checkup with the pediatrician. I can't wait to find out how much she has grown...she is just getting so big.

On a roll...

Elsa is rolling again. Yesterday, she rolled over in her crib once. Today, she rolled over in her crib twice and also rolled over once while playing. She is exactly 4 months and one week. I never did mention that Elsa is starting to hold her own with Sophie. She can actually make Sophie cry!!! Sometimes, when Sophie lays her head on Elsa's chest, Elsa will grab Sophie's bow along with a fistful of hair. Maybe Sophie will stop poking Elsa in the face so much (yea right). Sophie is just so fascinated with face parts. When she wakes up in the morning and also when she lays down for naps or bedtime, she points at all my face parts and wants me to label it. She will point to my eyebrows 5 times in a row...I think that is part of her hair obsession.

This past Tuesday was Haley's last day of school...yippee for summer breaks!!! Sophie and Elsa will get to spend a lot more time with their big sissy. We will have to plan a lot of fun summer activities soon because the summers seem to go by way too quickly.

Yesterday, Sophie, Elsa, Miranda (our cousin) and I went to a park that has all kinds of farm animals. Sophie absolutely loved it. She is so fascinated with pigs. She signs pig at least 20 times a day. Maybe she just likes to point to her nose and snort...who knows what goes through that girl's head! Grandma Debbie took her there a couple weeks ago so I was trying to figure out how much Sophie actually remembered. It seemed she somewhat knew her way around. I would love to take her to a farm where she could actually pet all the animals. At this place, there were signs all over saying the animals bite so I made sure we didn't get too close.

Talking...

I really think I can officially say that Sophie has moved beyond babbling and is now talking. Last week, she said eeebaa!!! That is huge!!! She strung 2 different sounds together and said it at the same time. She said it about 20 times that day but now will only occasionally say it. Then, the following day, she said baby. I didn't hear it but she said it for Grandma Debbie and Daddy. I think she said it about 3 times but I haven't heard it since. Then the following day, she said baaeeebee!!! That is a 3 syllable word. Okay, maybe it is not a real word but 3 different syllables nonetheless. Now she is saying ball (though the "ll" sound at the end isn't always clear). If you hold a ball up and ask her what it is she will say ball. Actual sound...no signing and no prompting. She is getting it!!! I am so proud of her. She is going to be talking up a storm before we know it. Woohoo!!!

By the way, we are now down to 125mg of Depakote twice a day. Her seizures are a bit stronger than before the wean and on some days she may have a few more than usual but isn't it worth it??? She was sacrificing development for a bit more seizure control. Isn't that a tough decision to make??? On one hand, I hate seeing these stronger seizures. On the other hand, I am ecstatic over her leaps and bounds on communication. Is this selfish on my part? No, I am her mother and I am only doing what I think is in her best interest (or at least...that is what I tell myself). Well, we have a neuro appointment next Tuesday, so I will update on what we are to do about this never ending seizure battle. By the way, May 10th was 2 years to the day when Sophie was diagnosed with Infantile Spasms. 2 years!!! I can remember that day like it was yesterday...ugh!!!

I just couldn't end this post on a negative note with it being such great news, so I will share a little story that filled my heart with joy today... Sophie was giving Elsa kisses today, like she always does. She loves to give her kisses and Elsa loves to get them. They entertain each other so much. Sophie will laugh in Elsa's face and Elsa will laugh right back at her. Well, after Sophie gave Elsa one of her big smooches, Sophie turned around and gave me one. Not that she has never done that before. But right at that moment, I saw the big picture....my little girl spontaneously giving me a kiss...how could life get any better.

Developmental milestones...

Elsa is my little developmental milestone overachiever. She is not even 4 months yet and she is doing so much. She loves following Sophie with her eyes. She is always interested in what she is doing. When Elsa is laying on her back, she will turn herself in circles to keep an eye on Sophie. She does this by pulling her legs up to her chest, rolling over to her side and then throws her head way back. By the time she has done this 4 or 5 times, she has made a complete circle. Her head control is absolutely amazing and she can push herself pretty high when on her belly. She also brings both hands to midline, clasps them together and then raises them above her head. She will grasp objects dangling above her head. When given a toy, she will bring it to her mouth. She loves to chew on toys and her hands. She is my little talker...a true Widmann!!! She loves playing with her toes and tries to bring them to her mouth. She bears weight on her legs. She smiles and laughs. She recognizes her immediate family and cries when strangers get too close.

Before Elsa, I hated the developmental milestone chart. It was always upsetting to read them. Even though Sophie continues to develop, she has always been about 10 - 14 months behind on the charts. So you can say, I have a love-hate relationship with the chart.

New signs...

Sophie is now signing "on", "take a break", and "blanket". It is such a collaborative effort and it is so exciting when you are the one that teaches her the new sign. Grandma Debbie taught her "on". I tried teaching her that one but I wasn't very effective. Grandma Debbie was able to teach it to her in a matter of hours or maybe even less than that. Sophie's ABA therapist taught her "take a break" as part of her therapy sessions. She is not quite there yet but will try very hard when verbally prompted. I am not exactly sure how she learned "blanket". It is part of her Baby Einstein - My First Signs video which she loves to watch. Occasionally, we will sit down together and I will assist her in doing the signs but I never reinforced "blanket". I think she just gets it now and picks up signs quite easily. So that is a total of 23 signs! That is pretty impressive considering she just started signing about 3 months ago.

Random pictures...

Here are pictures from random events over the past month...

Tummy time.
(Elsa)

More tummy time.
(Elsa)

Oh, so sweet!
(Elsa & Sophie)

Hair obsessed Sophie!
(Sophie & Elsa)

Holding hands.
(Sophie & Elsa)

Family portrait on the day of Elsa's baptism.

(Colin, Aunt Erica, Uncle Ed, Ben, Aunt Erin, Nicholas, Uncle Joe & Elsa)

Cousins.
(Nicholas & Elsa)

Sophie loves when her cousins drive her around in this jeep. It is one of her favorite things to do.
(Sophie & Colin)

Snapshot.
(Uncle Ed & Sophie)

Sisters...Can't get any better than this!!!
(Elsa, Sophie & Haley)
I love my big sis!
(Haley & Elsa)

Pictures taken on Mother's Day...

Big smiles.
(Elsa)

My mischievous little Sophie.

I love Daddy!
(Elsa & Brandon)

Kisses!
(Sophie & Zach)

Sophie playing with her own eyelashes.
(Sophie, Uncle Jason, Elsa & Aunt Colette)

My girls.
(Mommy, Sophie & Elsa)

Cuties.
(Sophie & Elsa)

Using signs...

Sophie is really starting to use the signs she has learned without being prompted. It all started with her pointing at everything she wanted. Then she learned her first sign which was "more". We had to use hand over hand to get her to do the sign. Then it went to us verbally prompting her to sign "more". Now she will sign "more" without us even prompting her. The other day, we were out shopping and Sophie started signing "more" and "drink" on her own!!! How exciting is that!!! She was actually telling me she was thirsty without visually seeing her cup. I was holding her in my arms and I was pushing Elsa in the stroller and she got my attention and did those signs. Even more exciting is that she is doing 2 signs in a row!!! She is doing all of this despite seizing all day long. That is one determined little girl!!!

Decrease in medication...

I spoke with Sophie's neurologist on the phone today. We are going to start weaning Sophie off all medications starting with Depakote. She is still having daily seizures and it does not seem the medication is helping at all. I would love to have her off all medication and see how that effects her behavior. Hopefully, she will tolerate the wean and we will not see an increase in seizures. If all works out as planned, we will then have a great baseline of her behavior and development before starting any new medications. Since it seems nothing is going to stop these seizures, the most important thing to me is Sophie's development.

More on Elsa...

Elsa is doing wonderful. She is our calm, laid back baby. It did not start out that way...she use to cry when Sophie would make her loud noises (which was very often), she startled very easily, it was hard to figure out when she was hungry or tired. She has gotten use to Sophie - licking and all!!! She is in a great routine and is sleeping for longer stretches during the night. She hardly ever cries. She entertains herself easily. She goes to sleep easily.

Elsa's accomplishments...she has rolled over from her tummy to her back a few more times, she is attempting to roll from her back to her tummy, she stares at her hands at amazement (just started doing that today), she has laughed a few times*, she is grasping objects and bringing them towards her, she is tracking objects (she actually started doing that at around 6 weeks), she has great control of her head and can even hold it up for several minutes at a time while she is on her belly, no flat spots (woohoo - you can only imagine how obsessive I was over that considering all that we went through with Sophie!!!), normal EEG (yes - Brandon and I freaked out about how much she would startle that we took her to get an EEG**), she is very talkative - especially when you talk to her, she loves to chew on her blanket and her fist (she is definitely going to be a blankie baby). Most importantly, she is just a happy and healthy little baby girl. I am trying so hard to take in every moment which is sometimes very hard to do but I occasionally remind myself to take a step back and just enjoy her. She is so very precious and has a smile that just melts my heart.

*One time Sophie laughed in her face and Elsa laughed right back at her...it was so precious!
** The startle reflex looks very much like a tonic seizure.

Sign, Sign, Everywhere A Sign...

Sophie is learning so many new signs. Brandon and I tried to list them all out tonight but she has so many that it is hard to remember. So here goes...

• More
• Eat
• Drink
• All done
• Up
• Down
  
• Help
• Hug
• Bath
• Bubbles
• Milk
• Pig
• Dog
• Baby
• Cry
• Open*
  
• Play*
  
• Daddy*
• Tree*

*She is still working on these. It's close to the actual sign but she does her own version of it.

In addition, she also waves hi and bye, blows kisses, and puts her hands up for so big. Her receptive language still continues to amaze me. She truly does understand everything we say to her. We are at the point where we have to spell things out so she doesn't know what we are talking about. The minute she hears the word "bath", she is dragging you off to the bathtub. Just tonight, I told Brandon that I did not give Sophie her vitamins yet. With that, she climbs off the bed and heads directly towards the cabinet in the kitchen where her vitamins are at.

Go Elsa!!!

Elsa rolled over from her tummy to her back twice today!!! She is not even 3 months old yet!!!

Outdoing each other...

Sophie spontaneously did a new sign today. I showed her the sign for "help" a few times but never pushed it. Today, during therapy, her therapist asked Sophie if she needed help and she signed "help" without being prompted. We were both surprised and thrilled. Not to be outdone by Sophie, Elsa decided to almost roll over today. She is also able to hold her held up real high for about 15 seconds. I will take this competition any day!!!

Time flies...

I am trying so hard to keep up with this blog. I just realized it has been 2 weeks since I last posted!!! 2 weeks...really?!? I seriously thought it was just last week. All my girls are doing incredible.

Haley...Haley won double platinum in her dance competition last weekend. Way to go Haley!!!

Elsa...Elsa is growing so fast. She loves cooing and is already able to hold her head up for 3 seconds while on her belly. She is also taking the bottle again - woohoo!!!

Sophie...Sophie's development continues to amaze us all. She will repeat upon command the following sounds - la la la, mew, ho ho ho, mmm, doi doi doi, aah, and ba. That is 7 different sounds!!! She makes more sounds than that but these are the ones she will repeat upon command. She is climbing up on things like crazy. She climbs up on the tall stools at the kitchen counter and then onto the counter. Just today, Brandon found her standing on top of her changing table and throwing things off our dresser. She gets into EVERYTHING. She is like a tornado when she enters a room. She loves to scatter her books and toys everywhere. I also find the oddest things in the oddest places. Just when I cannot imagine loving her anymore than I possibly can she finds a way to melt my heart all over again. She gives the most amazing hugs. She will wrap her little arms so tightly around my neck. It's the greatest feeling in the world.

Lots of pictures...

Sophie, Daddy and Elsa...What an exhausting day!!!

Sophie, Mommy and Elsa...Sophie loves to touch and hold ANYONE'S hair when she sucks her thumb. All she needs is the littlest amount of hair and that thumb goes in her mouth.

Elsa and Sophie...Sophie is very big into licking things nowadays. Unfortunately for Elsa, she loves to lick her head. Sticking her fingers into Elsa's mouth was just a little added bonus ;)

Sophie stealing Elsa's pacifier. Oh well, Elsa refuses to take the pacifier anyway (or a bottle right now - I'm in big trouble with that one).

Elsa and Haley...Elsa is already looking up to her big sister.

Sophie is trying to reach the cheese puffs on the top shelf.

Sophie loves climbing on everything and anything. She watched Haley climb up a slide once and she was determined to do it herself.

Despite the snow in the background, it was actually a nice day outside. Sophie walked from our back patio, down a couple of steps, and followed the grassy area where the snow melted around the house to get to her swing. She hates the snow. She did this all by herself. That took some logic for her to figure out how to get to her swing without walking in the snow.

Time to go in.

Here are pictures from Sophie's last hospital stay. Here she is oblivious to what is about to happen to her. Just hanging out in her pajamas and snacking on some trail mix.

Where do I begin...

I have not posted in so long about Sophie's development. She is doing INCREDIBLE!!! She does something new every single day!!! She just amazes me.

Physically...Her balance is getting so much better. She climbs on anything and everything which is very scary at times. She is able to go up and down the stairs by herself. She usually walks up the steps by holding onto the spindles or bracing herself against the wall (in other words she is no longer crawling up the steps) and she usually goes down the stairs by sliding on her butt which is good for safety issues. If she is out somewhere else and it is only a few steps, she can walk up them without holding on to anything and if there is something to hold on to she will try walking down the steps. She is also able to pedal on her tricycle, kick a ball around and bounce on her trampoline.

Cognitively...She understands almost everything we say to her. It amazes me everyday of how much she understands. I will tell her to do something that I did not specifically teach her and she will do it. She is picking up on things just by hearing us talk. Though she does not seem to understand what we are saying to her when it is something she does not want to do - that is how much she understands. On the other hand, it is sometimes hard for us to understand what she wants. That is where the frustration comes in - for all of us. Sophie is starting to throw temper tantrums which is very age appropriate! Most of her temper tantrums revolve around food. She knows exactly where her snacks are in the pantry and she does not take no for an answer. Also, at dinner, it is sometimes hard to figure out what she wants to eat even though she is an incredible eater. She eats almost anything. But if you try to give her a bite of something that she does not want at that moment, she can have a complete meltdown.

Fine motor skills...Just a few days ago, she started putting her plastic coins into her piggy bank. That takes a lot of fine motor skills to line up the coin just the right way to drop into the slot. Of course, we have been working with her on this for quite some time and Sophie just so nonchalantly walks over and just starts doing it on her own. That is Sophie for you. She loves to do things out of the blue on her own terms.

Speech...She is learning sign language. Her first real sign was "more". She uses the same sign for eat and drink so we are working on changing her sign for drink. She can sign bubbles, baby, cry, all done and milk. I am sure I am probably missing a few. She also does some signs for which I do not understand. I am not sure if she is starting to make them up herself or if it is things she is trying to imitate but she is just not doing it correctly. It is so wonderful that we are starting to be able to communicate without having to point at everything. She is also starting to imitate sounds. When asked what a cat says, she will say "mmmw". Not quite meow but close. She will also repeat "lalalala" when asked.

This post does not do Sophie justice when it comes to her development. The list can just go on and on. She has just improved leaps and bounds. It is all the little things she does and understands that amazes me.

First IVIG treatment...

Sorry I did not post sooner. We left for the hospital at 6:45am on Wednesday morning and did not get home until 11:00am on Thursday. Needless to say, it was a very long 28 hours. We were only suppose to be there until late afternoon on Wednesday.

Sophie handled her first infusion fairly well...just a little nauseous. It was getting the leads for the EEG and the IV started that was the hard part. She screamed for at least an hour straight. The nurses kept commenting on how strong she is. By the time they actually started the infusion, it was 11:00am. Since this was her first infusion, they wanted to do the infusion at a slow rate which would take about 9 hours. Well, when the doctor came by around 4:00pm on Wednesday, he suggested we stay the night since we were going to be here so late anyway. He wanted her to be observed for a few hours after the infusion and also wanted to collect further readings on the EEG. Since the hard part was already done, we agreed.

When we got home on Thursday, I gave her bath, she ate some lunch and then took a nap. By the time she woke up from her nap, she was back to her usual self. She is such a trooper. If only I could bounce back so quickly. She just amazes me. Only time will tell if this treatment will work as it takes awhile to build up the immunities. I will keep you all posted.

Thank you all for your thoughts, prayers and kind words. Thank you especially to Grandma Debbie for watching Elsa and coming to the hospital on Thursday. Thank you Uncle Jason and Aunt Colette for coming by the hospital so Elsa would not starve (Brandon tried giving Elsa formula on Thursday and she refused to drink it but luckily we arrived home soon after) and bringing us lunch.

IVIG update...

I decided to matters into my own hands. Last week, I made an appointment with a pediatric hematologist. We met with him last Wednesday and he said we could start the IVIG treatment as soon as possible. By Thursday, I had a call from his office saying everything was coordinated with the hospital in order for her to get the IVIG treatment and EEG. Our appointment is tomorrow morning!!! Can you believe that?!? Nothing ever happens that quickly...especially without several phone calls.

The first treatment would take all day since it would be given slowly to see how Sophie reacts. If she tolerates the first treatment well, the rest of the treatments will be much quicker. Usually, they give the treatments in the office. Since their office is in a pediatric cancer center (very heartbreaking seeing those kids there), it is setup for such treatments with a big play area and televisions. But, since I want Sophie to also have an EEG at the same time (to see how her brain reacts to the treatment), we had to coordinate efforts with the hospital and she will receive her first treatment in the hospital.

Please keep Sophie in your thoughts and prayers tomorrow. It is going to be a very traumatic day for her since she will be confined to a hospital bed with numerous leads (for the EEG) glued to her head along with an IV. But as always, I know she will be a trooper and be back to her happy self by the time we get home.

It is so hard for me to keep up with this blog...emotionally and physically. We are still in limbo regarding IVIG treatments as we have to meet with a neurologist at the Epilepsy Center at Children's Hospital as our regular neuro is not familiar with IVIG treatment. Of course, nothing is ever easy. It took 3 phone calls to the Epilepsy Center and a phone call to our regular neuro before we finally got an appointment. When the receptionist at the Epilepsy Center finally called us back with an appointment, the earliest appointment they could give us was April 21st. So then I had to call our regular neuro again since he said they should be getting us in within the next couple of weeks NOT in a couple of months. I am still waiting to hear back from our regular neuro. Of course, nobody is in a rush to get things done because it's not THEIR CHILD HAVING DAILY SEIZURES!!! Can you tell I am frustrated??? I am just so tired. Tired of always being Sophie's advocate. Tired of fighting. Tired of researching new treatment options. Tired of watching Sophie having seizures everyday. Tired of all the therapy sessions. Tired of not living a normal life. Tired of wondering what Sophie's future will be like. Tired of working with Sophie everyday to get her to do things that she should have been doing a year ago. Tired of feeling guilty. But Sophie keeps me going. That precious smile of hers. She is just so incredibly adorable. I love her so much.

Why I haven't posted...

I have not posted in the last month because on January 7, 2008, Sophie started having seizures again. She has been having seizures every day since then. We have tried increasing her Depakote with no success. I am sorry to all of those who I have not been honest with in the last month. I did not want to admit to myself or to anyone else that her seizures returned. By saying it out loud it made it more real. I was in denial. I thought with an increase in medication that they would go away again and I could forget that they ever returned. Unfortunately, they have not gone away and I can no longer pretend that they did not come back.

Things have been crazy this last month between Sophie's seizures and getting ready for the new baby. Elsa Kate was born on January 22nd and is a healthy baby girl...weighing in at 8 pounds 6 ounces and is 21 inches long.

We are currently researching IVIG treatment for Sophie's next treatment option. If anyone has any experience with IVIG, please let me know.

2007 - what a year...

To sum up 2007 -

• 5 ER visits
• April 2nd - hit her head on the tv stand and cut her face above her left eye resulting in 5 stitches
  
• October 20th - fell out of bed and cut her face above her right eye resulting in dermabond
  
• October 24th - started wheezing in the middle of the night and was diagnosed with a severe case of croup
  
• November 19th - fell down the basement steps resulting in a big knot on her forehead but luckily nothing else
  
• November 30th - sick and acting very strangely and showing symptoms of possible adverse drug reaction but turned out to be some kind of virus
  
• 1 minor surgery
• March 8th - ear tubes (yeah - no ear infections ever since and this is after being on 6 rounds of antibiotics in 3 months)
  
• Numerous trips to Chicago for her cranial molding helmet
• Between January through June - about 13 trips to Chicago (though we did enjoy getting to spend more time with Aunt Erica, Uncle Ricky and their cat Sylvester)
  
• Numerous EEGs (I have lost count but each one continues to look better than the last)
  
• 2 trips to Johns Hopkins Hospital in Baltimore
• April 15th - admitted for a week to start the ketogenic diet
• July 17th - follow up visit
• Several medication changes
• January 18th - started clonazepam
  
• February 2nd - started clobazam
• February 2nd - stopped clonzaepam
• February 14th - stopped depakote
• April 15th - started ketogenic diet
  
• May 14th - stopped zonegran
  
• September 7th - started keppra
• September 22nd - stopped keppra
• September 27th - started weaning diet
  
• November 10th -started 2nd round of depakote
• Developmental progress
  
• January - pulled herself to a standing position
• January - started using pointer finger to push buttons
• February - started crawling from one room to another
  
• February - started walking with push toy
• February - started cruising around furniture
• April - took first steps without assistance
• April - started climbing steps
• May - took about 10 steps without falling
• August - started using pointer finger to communicate what she wants
• August - started babbling more
• October - started putting objects in a container
• November and December - so many it is hard to list everything...her development is at an all time high (I will post again soon with all the new things she is currently doing)
  
• Last but not least...
• 28 DAYS OF NO SEIZURES AND STILL COUNTING!!!

What a wonderful way to end 2007!!! Though, we almost ended up in the ER on New Year's Eve when Sophie tried to eat a glass ball ornament. Luckily, we caught her trying to do this before she had a chance to swallow anything. She had 2 pieces in her mouth which matched up perfectly to what was missing on the ornament. Sophie sure loves keeping up the drama. Happy New Year!!!

27 days and counting...